Madison 19yrs old  Bailey Kruse 2005 (5 y/o) - Princess B

Chorea Return to Index
Definition:
  • Ceaseless rapid complex body movements that look well coordinated and purposeful but are, in fact, involuntary (from MedicineNet.com).
  • Any of various disorders of the nervous system marked by involuntary, jerky movements, especially of the arms, legs, and face, and by incoordination (from Answers.com).
  • Uncontrolled, purposeless, rapid and jerky movements of face and body. Frequently observed movements are facial grimacing, raising the shoulders and flexing and extending the fingers (from Knowledgerush.com).
Occurrence in FA:
  • Very rare and mostly connected with point mutation FA.
  • 2002 article about a one point mutation FAer (2% to 4% of the FA population) that had chorea.
  • 2004 article about two Malaysian siblings with a rare form of FA that included chorea.
  • The above two reports are the only FAPG/INTERNAF reported FA with chorea in thousands of Emails since 1998.
  • In a 2004 NAF conference presentation, Dr. John Day talked about mitochondria inherited conditions. He split them into two categories:
    • Nuclear DNA Defects (FA) (Nuclear-encoded problems: FA, Wilson, Hereditary spastic parapalegia, ALS)
    • Mitochondrial DNA defects (Mitochondrial mutations: parkinsons, dystonia, chorea, ataxia [neuropathy])
  • In summary, if an ataxian displays chorea symptoms it is unlikely the source is part of the classic two-bad-gene Friedreich’s Ataxia; rather another ataxia or disorder source is likely.

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